ICD-11 code : 4A43.1;
Preferred Label : Mikulicz disease;
ICD-11 definition : Mikulicz disease is a disorder first reported by Johann von Mikulicz in 1892 and characterized
by symmetrical swelling of the lachrymal, submandibular, and parotid glands, with
massive infiltration of these glands by mononuclear cells. Serum autoantibodies, such
as anti-Ro/SS-A, are usually negative and serum IgG4 concentration may be increased.
Unlike Sjögren disease, IgG4-Mikulicz disease is characterized by the formation of
lymphoid follicles, but shows lower levels of lymphocytic infiltration into salivary
ducts, such that their structure remains intact.;
Origin ID : 2012865457;
Automatic exact mappings (from CISMeF team)
- ICD-10 Mapping
Mikulicz disease is a disorder first reported by Johann von Mikulicz in 1892 and characterized
by symmetrical swelling of the lachrymal, submandibular, and parotid glands, with
massive infiltration of these glands by mononuclear cells. Serum autoantibodies, such
as anti-Ro/SS-A, are usually negative and serum IgG4 concentration may be increased.
Unlike Sjögren disease, IgG4-Mikulicz disease is characterized by the formation of
lymphoid follicles, but shows lower levels of lymphocytic infiltration into salivary
ducts, such that their structure remains intact.
